Two Unusual Cases of Giant Cell Myocarditis Associated With Mitral Stenosis and With Wegener's Syndrome

McCrea, P. C.; Childers, Rory

doi:10.1136/hrt.26.4.490

Cited by 42 publications

(16 citation statements)

References 15 publications

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“…As in previously reported cases, 14,16,18 we confirmed an association with rheumatic valve disease in some patients, suggesting related pathogenetic (possibly immune) mechanisms. It has long been recognized that granulomatous inflammation may be seen in atrial appendages excised from patients undergoing mitral valve replacement for rheumatic mitral stenosis.…”

Section: Etiology and Pathogenesissupporting

confidence: 74%

Atrial Giant Cell Myocarditis

et al. 2013

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Background— Giant cell myocarditis (GCM) typically causes fulminant heart failure, arrhythmias, or heart block, necessitating aggressive immunosuppression, ventricular assist device insertion, or cardiac transplantation. We describe a novel variant of GCM, primarily involving the atria, that displays distinctive clinical features and follows a more benign course than ventricular GCM. Methods and Results— We identified 6 patients (median age 67.5 years, 4 male) with atrial GCM in our pathology consultation practices from 2010 to 2012. Clinical history, imaging, and pathology materials were reviewed. Clinically, 4 patients had atrial fibrillation, 1 had acute heart failure, and 1 had incidental disease at autopsy. Among the 5 living patients, echocardiography revealed severe atrial dilatation (5 cases), mitral/tricuspid regurgitation (5), atrial mural thrombus (3), atrial wall thickening (2), and atrial hypokinesis (2). Ventricular function was preserved in all 5. Histological review of surgically resected atria showed giant cell and lymphocytic infiltrates, lymphocytic myocarditis-like foci, cardiomyocyte necrosis, and cardiomyocyte hypertrophy in all cases. Other features included interstitial fibrosis (5), poorly-formed granulomas (4), eosinophils (4), neutrophils (1), and vasculitis (1). Treatment consisted of steroids and cyclosporine (1), pacemaker placement for sick sinus syndrome (1), and supportive care (3). All 5 living patients returned to baseline exercise tolerance after 6 to 16 weeks of follow-up. Conclusions— Atrial GCM represents a distinct clinicopathologic entity with a more favorable prognosis than classic ventricular GCM. This disorder should be included in the differential diagnosis of atrial dilatation, particularly when associated with atrial wall thickening. The utility of immunomodulatory therapy for this condition remains unknown.

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Section: Etiology and Pathogenesissupporting

confidence: 74%

Atrial Giant Cell Myocarditis

et al. 2013

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“…On the basis of pathological examination, myocarditis has been reported to occur in 25% of cases [7]. Moreover, giant cell myocarditis (a fulminant form of myocarditis) has been correlated with WG [23, 24, 25]. WG involving the myocardial tissue and presenting as a ventricular mass has also been described [26].…”

Section: Myocardial Diseasementioning

confidence: 99%

“…The most commonly encountered disorders are aortic regurgitation, mitral valve thickening, mitral valve prolapse and regurgitation, and mitral stenosis [5, 6, 10, 14, 21, 23, 31, 32, 33, 34, 35, 36]. These abnormalities may be primary, due to valvulitis, or secondary, due to myxomatous degeneration of the valve, or dilatation of the valve ring or left ventricle [5, 6, 33].…”

Section: Valvular-endocardial Diseasementioning

confidence: 99%

The Heart in Wegener’s Granulomatosis

2004

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Wegener’s granulomatosis (WG) is a necrotizing vasculitis that mainly affects the respiratory tract and kidneys. Of note, involvement of the heart is being increasingly recognized in these patients. Cardiac manifestations can arise from the coronary arteries, pericardium, myocardium, endocardium, valves, conduction system and great vessels, but in most cases cardiac involvement is clinically silent. Consequently, a regular cardiovascular evaluation, including echocardiography, should be performed in every patient with WG, while patients with WG flare should be closely monitored for cardiac complications even in the absence of specific symptoms.

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“…Rarely giant cells may be seen in syphilitic myocarditis [73]. Foreign body reaction, Wegener's granulomatosis [54,58], and systemic sarcoidosis must be considered in the differential diagnosis as well. These disorders have distinct clinical presentations and appropriate diagnostic studies usually preclude confusion with idiopathic giant cell myocarditis.…”

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confidence: 99%

Giant Cell Myocarditis: Diagnosis and Treatment

Cooper

2000

Herz

109

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Giant cell myocarditis is a rare but devastating disease that usually affects young otherwise healthy individuals. Associations with thymoma, inflammatory bowel disease, and a variety of autoimmune disorders have been reported. The rate of death or heart transplantation is approximately 70% at 1 year. Data from a Lewis rat model and from observational human studies suggest that giant cell myocarditis is mediated by T lymphocytes and may respond to treatment aimed at attenuating T cell function. Recent findings from the Giant Cell Myocarditis Registry, a clinical and pathologic database from 63 cases of giant cell myocarditis gathered from 36 medical centers, include the following: The sensitivity of endomyocardial biopsy for giant cell myocarditis for patients who undergo transplantation or autopsy is 82 to 85%. Registry subjects who received cyclosporine in combination with steroid, azathioprine, or muromonab-CD3 have prolonged transplant-free survival (12.6 months vs. 3.0 months for no immunosuppression). Post-transplantation survival is approximately 71% at 5 years despite a 25% rate of giant cell infiltration in the donor heart. To confirm and extend these findings, a randomized trial of immunosuppression including muromonab-CD3, cyclosporine, and steroids is underway.

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Two Unusual Cases of Giant Cell Myocarditis Associated With Mitral Stenosis and With Wegener's Syndrome

Cited by 42 publications

References 15 publications

Atrial Giant Cell Myocarditis

Atrial Giant Cell Myocarditis

The Heart in Wegener’s Granulomatosis

Giant Cell Myocarditis: Diagnosis and Treatment

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